eMedicine Specialties > Pediatrics: General Medicine > Hematology

Transient Erythroblastopenia of Childhood

Author: Lennox H Huang, MD, Associate Chair (Clinical) of Pediatrics, Assistant Professor of Pediatrics, McMaster University, Deputy Chief, McMaster Children's Hospital, McMaster Children's Hospital
Coauthor(s): Carol Portwine, MD, Division of Paediatric Hematology and Oncology, Assistant Professor of Pediatrics, McMaster Children's Hospital; Robin Miller, MD, Assistant Professor, Department of Pediatrics, Rainbow Babies and Children's Hospital, Division of Hematology-Oncology, Case Western Reserve School of Medicine, University Hospitals of Cleveland
Contributor Information and Disclosures

Updated: Aug 28, 2007

Introduction

Background

Transient erythroblastopenia of childhood (TEC) is a slowly developing anemia that occurs in early childhood and is characterized by a gradual onset of pallor. As the name suggests, all patients with TEC recover completely without sequelae.

Pathophysiology

The etiology of TEC is unknown. However, researchers have proposed numerous viral and immunologic mechanisms. At least 2 separate case reports have noted pure red cell aplasia with concomitant human parvovirus B19 infection.1,2 However, a prospective case series of 10 patients failed to identify a single viral causative agent for TEC.3

In vitro studies using serum and immunoglobulin G (IgG) from some patients with TEC demonstrated erythroid colony suppression, suggesting an immunologic etiology. TEC is not caused by a lack of erythropoietin. Bone marrow from patients with TEC exhibits an absence of red cell precursors.

Frequency

United States

Attempts to determine frequency of TEC are limited by an unknown number of asymptomatic undiagnosed cases.

Mortality/Morbidity

Morbidity relates to the severity of the anemia and diagnostic workup.

  • Children with TEC have reportedly presented with high-output shock secondary to profound anemia.4  
  • Patients with atypical TEC may require invasive tests such as bone marrow aspiration or biopsy.
  • Association of transient neurologic deficits may lead the physician to pursue CNS imaging studies or a neurologic consultation.

Sex

The male-to-female ratio is 1.4:1.

Age

The median age of presentation is 18-26 months; however, the disorder may occur in infants younger than 6 months and in children as old as 10 years. In contrast, Diamond-Blackfan anemia tends to present in child younger than 1 year, whereas human parvovirus B19–associated erythroblastopenia typically presents at an older age.

Clinical

History

  • Most individuals with transient erythroblastopenia of childhood (TEC) present with gradually increasing pallor and no other symptoms despite the severity of the anemia. 
  • Occasionally, parents report increased fatigue or decreased energy levels in children with TEC. 
  • Some isolated incidents of transient neurologic events and breath-holding spells have been reported in association with TEC. 
  • Other differential considerations (eg, aplastic crises, hyperhemolytic crises, sequestrations) typically present more acutely than TEC. Fatigue and pallor develop over the course of days and are often associated with nonspecific viral symptoms, such as fever, malaise, lethargy, abdominal pain, or upper respiratory symptoms. Jaundice may also be a presenting symptom, especially in the context of a preexisting hemoglobinopathy such as sickle cell disease or hereditary spherocytosis.

Physical

  • Upon physical examination, patients are usually healthy except for findings commonly associated with anemia, such as skin and mucosal pallor, tachycardia, and, often, a cardiac flow murmur. 
  • By contrast, the most common congenital anomalies associated with Diamond-Blackfan anemia include short stature, low birth weight, developmental delay, thumb malformations, craniofacial anomalies, and urogenital abnormalities. Examining for physical anomalies is important because they are found in as many as 70% of patients with Diamond-Blackfan anemia. 
  • A complete neurologic examination is necessary because of case-report associations.
  • In patients with symptoms such as splenomegaly and icterus, consider other diagnoses such as a hemolytic-associated anemia or sequestration-associated anemia. A characteristic "slapped cheek" rash is often associated with parvovirus B19 infection and aplastic anemia.

Causes

  • The cause of TEC is unknown. Viral and immunologic mechanisms may be involved. Reports of seasonal clusters of incidents of TEC, although suggestive of a viral etiology, are not statistically significant. Only a handful of familial TEC cases have been reported, and no apparent genetic link has been elucidated.

More on Transient Erythroblastopenia of Childhood

Overview: Transient Erythroblastopenia of Childhood
Differential Diagnoses & Workup: Transient Erythroblastopenia of Childhood
Treatment & Medication: Transient Erythroblastopenia of Childhood
Follow-up: Transient Erythroblastopenia of Childhood
References
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References

  1. Prassouli A, Papadakis V, Tsakris A, et al. Classic transient erythroblastopenia of childhood with human parvovirus B19 genome detection in the blood and bone marrow. J Pediatr Hematol Oncol. Jun 2005;27(6):333-6. [Medline].

  2. Geetha D, Zachary JB, Baldado HM, et al. Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature. Clin Transplant. Dec 2000;14(6):586-91. [Medline].

  3. Skeppner G, Kreuger A, Elinder G. Transient erythroblastopenia of childhood: prospective study of 10patients with special reference to viral infections. J Pediatr Hematol Oncol. May 2002;24(4):294-8. [Medline].

  4. Chabali R. Transient erythroblastopenia of childhood presenting with shock and metabolic acidosis. Pediatr Emerg Care. Oct 1994;10(5):278-80. [Medline].

  5. Chan GC, Kanwar VS, Wilimas J. Transient erythroblastopenia of childhood associated with transient neurologic deficit: report of a case and review of the literature. J Paediatr Child Health. Jun 1998;34(3):299-301. [Medline].

  6. Cherrick I, Karayalcin G, Lanzkowsky P. Transient erythroblastopenia of childhood. Prospective study of fifty patients. Am J Pediatr Hematol Oncol. Nov 1994;16(4):320-4. [Medline].

  7. Freedman MH. ''Recurrent'' erythroblastopenia of childhood. An IgM-mediated RBC aplasia. Am J Dis Child. May 1983;137(5):458-60. [Medline].

  8. Gussetis ES, Peristeri J, Kitra V, et al. Clinical value of bone marrow cultures in childhood pure red cell aplasia. J Pediatr Hematol Oncol. Mar-Apr 1998;20(2):120-4. [Medline].

  9. Gustavsson P, Klar J, Matsson H, et al. Familial transient erythroblastopenia of childhood is associated with thechromosome 19q13.2 region but not caused by mutations in coding sequencesof the ribosomal protein S19 (RPS19) gene. Br J Haematol. Oct 2002;119(1):261-4. [Medline].

  10. Krijanovski OI, Sieff CA. Diamond-Blackfan anemia. Hematol Oncol Clin North Am. Dec 1997;11(6):1061-77. [Medline].

  11. Miller R, Berman B. Transient erythroblastopenia of childhood in infants < 6 months of age. Am J Pediatr Hematol Oncol. Aug 1994;16(3):246-8. [Medline].

  12. Mupanomunda OK, Alter BP. Transient erythroblastopenia of childhood (TEC) presenting as leukoerythroblastic anemia. J Pediatr Hematol Oncol. Mar-Apr 1997;19(2):165-7. [Medline].

  13. Tam DA, Rash FC. Breath-holding spells in a patient with transient erythroblastopenia of childhood. J Pediatr. Apr 1997;130(4):651-3. [Medline].

  14. Walters MC, Abelson HT. Interpretation of the complete blood count. Pediatr Clin North Am. Jun 1996;43(3):599-622. [Medline].

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Further Reading

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Keywords

transient erythroblastopenia of childhood, TEC, anemia, pallor, pure red cell aplasia, human parvovirus B19, Diamond-Blackfan anemia, aplastic crises, hyperhemolytic crises, hemoglobinopathy, sickle cell disease, hereditary spherocytosis, splenomegaly, icterus, hemolytic-associated anemia, sequestration-associated anemia, aplastic anemia

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Contributor Information and Disclosures

Author

Lennox H Huang, MD, Associate Chair (Clinical) of Pediatrics, Assistant Professor of Pediatrics, McMaster University, Deputy Chief, McMaster Children's Hospital, McMaster Children's Hospital
Lennox H Huang, MD is a member of the following medical societies: American Academy of Pediatrics, Canadian Medical Association, Ontario Medical Association, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Carol Portwine, MD, Division of Paediatric Hematology and Oncology, Assistant Professor of Pediatrics, McMaster Children's Hospital
Disclosure: Nothing to disclose.

Robin Miller, MD, Assistant Professor, Department of Pediatrics, Rainbow Babies and Children's Hospital, Division of Hematology-Oncology, Case Western Reserve School of Medicine, University Hospitals of Cleveland
Disclosure: Nothing to disclose.

Medical Editor

Sharada A Sarnaik, MD, Director of Sickle Cell Program, Department of Pediatrics, Professor, Children's Hospital of Michigan and Wayne State University
Sharada A Sarnaik, MD is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Nothing to disclose.

Managing Editor

James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society
Disclosure: Nothing to disclose.

CME Editor

Samuel Gross, MD, Professor Emeritus, Department of Pediatrics, University of Florida, Clinical Professor, Department of Pediatrics, UNC, Adjunct Professor, Department of Pediatrics, Duke University
Samuel Gross, MD is a member of the following medical societies: Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA, Executive Director, Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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